Document Detail

Otologic manifestations of Hunter syndrome and their relationship with speech development.
MedLine Citation:
PMID:  18212495     Owner:  NLM     Status:  MEDLINE    
The excessive storage of mucopolysaccharide in Hunter syndrome leads to various otologic manifestations. We interviewed 19 patients with Hunter syndrome to assess their otologic problems, and conducted audiologic tests and temporal bone CT. Patients with the intermediate or severe form exhibited severe speech delay by more than 2 years (12/14 patients). However, in patients with the mild form (5/5), speech development was not much disturbed (2/5), although otoscopic findings were similar. The hearing threshold determined by the auditory brainstem response differed significantly between the mild and intermediate/severe forms (p < 0.05). Therefore, patients with the mild form may benefit from active otologic intervention such as VT insertion, amplification, and speech therapy.
Yang-Sun Cho; Joon Ho Kim; Tae Wook Kim; Soo Chan Chung; Son-A Chang; Dong-Kyu Jin
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2008-01-22
Journal Detail:
Title:  Audiology & neuro-otology     Volume:  13     ISSN:  1421-9700     ISO Abbreviation:  Audiol. Neurootol.     Publication Date:  2008  
Date Detail:
Created Date:  2008-04-10     Completed Date:  2008-08-06     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9606930     Medline TA:  Audiol Neurootol     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  206-12     Citation Subset:  IM    
Copyright Information:
(c) 2008 S. Karger AG, Basel
Department of Otorhinolaryngology - Head and Neck Surgery, Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul, South Korea.
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MeSH Terms
Child, Preschool
Ear, Middle / pathology
Hearing Disorders / etiology*
Language Development Disorders / etiology*,  physiopathology
Learning Disorders / etiology
Mastoid / pathology
Mucopolysaccharidosis II / genetics,  pathology,  physiopathology*,  psychology
Prospective Studies
Speech Intelligibility / physiology*

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